Bilateral pheochromocytomas and asynchronous bilateral renal cell carcinomas associated with von Hippel-Lindau disease: a case report

A 38-year-old man who had been followed with diagnosis of dilated cardiomyopathy and retinal angioma was referred to our hospital because of incidentally detected bilateral adrenal masses. Although he was normotensive, levels of catecholamine in urine were elevated and I131-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Screening of central nervous system by MRI revealed cerebellar hemangioblastoma. Right adrenalectomy and left partial adrenalectomy were performed, both of which tumors pathologically diagnosed as pheochromocytoma, followed by resection of the cerebellar hemangioblastoma. Five months later, abdominal CT revealed a left renal tumor and underwent left partial nephrectomy, being diagnosed as renal cell carcinoma. A right renal tumor was detected on follow up CT at 1 year after the partial nephrectomy. Since cardiac function was deteriorated, we have followed with careful observation. This is the second documented case of bilateral renal cell carcinomas and bilateral pheochromocytomas with VHL in Japan.     

A case of bilateral renal cell carcinoma

A case of bilateral renal cell carcinoma is reported. The patient was a 82-year-old male with a chief complaint of hematuria. Echogram, pyelogram and computed tomogram of abdomen revealed a left renal tumor, in addition right renal tumor also was found. Then, he underwent a radical right nephrectomy and a left partial nephrectomy. After the operation renal function was transiently impaired, but recovered two months later. The patient was followed up without any adjuvant therapy. Lung metastases were found at 1 year after the operation, and he died in spite of administration of alpha-interferon. In the present case, the bilateral renal tumors are presumed to have been caused by metastasis from contralateral tumor, since lung metastases were found.     

Solitary pancreatic metastasis from renal cell carcinoma

A case of asynchronous bilateral renal cell carcinoma with pancreatic metastasis is described. Left nephrectomy and tumorectomy in the right kidney were performed. Solitary metastasis to the pancreas without symptoms was treated by distal pancreatectomy with tumor and splenectomy. Although bilateral renal cell carcinomas were histologically renal cell carcinoma (clear cell subtype, grade 2), the resected pancreatic tumor was renal cell carcinoma with sarcomatoid change. Therefore, the metastatic tumor had a more malignant potential than the primary tumor. The pancreatic metastasis was seen at 6 years 10 months and 2 years 6 months after left nephrectomy and enucleation of the right renal tumors, respectively. The patient is alive without disease and is being treated by alpha-interferon for 12 months after distal pancreatectomy. A careful long-term follow-up of the patient with renal cell carcinoma seems to be necessary.     

Renal function and outcome following salvage surgery for bilateral Wilms' tumor

Purpose

Maintaining adequate renal function without compromising cure in children with bilateral Wilms’ tumor is surgically demanding and challenging. The aim of this study was to assess renal function and outcome in children treated in one institution for bilateral Wilms’ disease by renal salvage procedures.

Methods

This study is a retrospective review of all patients with histologically proven bilateral nephroblastoma who underwent a renal salvage procedure between November 1973 and June 2002. The median follow-up time was 52 months (range, 8 to 326 months).

Results

Twenty-three patients were included in the study. The median age at diagnosis was 19 months (range, 5 to 65 months). Patients who presented before 1982 (n = 5) were treated surgically first followed by chemotherapy. The remainder (n = 18) received initial chemotherapy before the operation. Of the 46 kidneys, 18 had a nephrectomy. The remaining 28 underwent a renal salvage procedure. At follow-up, 19 patients had good renal function, 2 had satisfactory function, and 2 had renal failure. Seventeen of the 23 children are alive and tumor free at follow-up (74%). Four children died of distant metastases and 2 of renal failure. Local recurrence in the salvaged kidney was detected in 1 patient 12 years after surgery after the commencement of immunosuppression for renal transplantation.

Conclusions

These results confirm that renal salvage procedures, in combination with chemotherapy, are a safe and effective way of treating children with bilateral Wilms’ disease. In addition, renal salvage surgery can maintain satisfactory renal function in the majority of these patients without an increased risk of local recurrence. When transplantation is required, the remaining native kidney should be removed to prevent tumor recurrence.     

双侧同时性肾癌治疗方式的选择

目的 探讨双侧同时性肾癌患者有效治疗方式的选择. 方法双侧同时性肾癌14例.男12例,女2例.平均年龄45岁.临床表现腰部酸痛感4例、血尿2例、体检偶然发现8例.14例术前影像学检查均明确为双侧肾脏占位性病变.其中发现腔静脉癌栓1例,肾门或腹膜后淋巴结肿大2例.14全均未发现远处转移.Robson分期Ⅱ期11例,Ⅲ期3例.肿瘤直径1.0～8.5 cm.行一期双侧手术6例,其中行双侧肾部分切除4例,一侧根治性肾切除、对侧肾部分切除2例.行二期手术8例,先行一侧部分切除术,1个月后行对侧根治性肾切除术3例;先行一侧根治性肾切除术,1个月后行对侧部分切除术2例;先行一侧部分肾切除术,1个月后行对侧肾部分切除术2例;1例伴有腔静脉癌栓患者一期于下腔静脉肝下水平放置静脉内支架加滤网防止癌栓脱落,再行根治性肾切除,1个月后再行对侧肾部分切除.结果 14例共行22例次手术,均获成功.一期双侧手术者平均手术时间(263±52)min;二期手术者平均手术时间(155±46)min.病理报告:29个肿瘤中,透明细胞癌23个,未分化肿瘤4个,嫌色细胞癌2个.10例患者获随访,随防时间6～41个月.3例行一侧肾部分切除加对侧根治性肾切除患者SCr升高,但无需血液透析;1例术后14个月后双侧肺转移,25个月后死亡;1例术后21个月左侧肾窝肿瘤复发,经放疗及化疗,38个月后死亡,1例术后6个月死于脑血管意外;6例无瘤存活,1例腔静脉癌栓未切除者随访32个月存活.结论 双侧肾肿瘤的处理原则为兼顾肾功能保留和肾肿瘤治疗,治疗方案应根据患者全身情况及肿瘤大小、部位进行详细评估,尽可能保留足够的肾功能.     

Bilateral Wilms' tumor

Recent studies indicate a favorable outcome with bilateral Wilms' tumor. From 1971 to 1985, ten children between the ages of 6 months and 5 years were treated for this disease. During the early part of the series, five patients had nephrectomy on one side and partial nephrectomy on the other side, and one patient had bilateral partial nephrectomies at the same operation. More recently four patients initially had a biopsy of both tumors and lymph node sampling followed by chemotherapy. At the second-look procedure two patients had multiple biopsies because there was no obvious tumor. Histologically there was no tumor on the third-look procedure in these two patients. Nine patients had a favorable histology of the Wilms' tumor. One patient had a favorable histology on one side and an unfavorable type of histology on the other side. Eight patients are surviving between 6 months and 13 years. Two died of extensive disease within 16 months of diagnosis. One patient had an unfavorable histology. The good results following partial nephrectomies have led us to attempt to conserve additional tissue, as has been done in the last four patients. Our early results suggest biopsy of the tumor followed by chemotherapy, then a second look, and if necessary, third-look procedures may result in preservation of functioning renal tissue.     

Bilateral adrenal masses from renal cell carcinoma: a case report

We report a case of bilateral adrenal metastasis from renal cell carcinoma. A 65-year-old man was referred to our hospital for a right renal mass. A computed tomography revealed a 9 cm right renal tumor and bilateral adrenal masses (3.5 cm on the right side and 4.5 cm on the left). A right radical nephrectomy and bilateral adrenalectomy demonstrated renal cell carcinoma with metastasis to bilateral adrenal glands. The pathological findings of the right renal tumor showed clear cell carcinoma, G3 > G2 and both adrenal tumors showed the same pathology as the right renal tumor. The patient is alive with lung metastasis after 15 months postoperatively treated with interferon-alpha.     

Case of bilateral renal cell carcinoma with the tumor thrombus in the inferior vena cava

We describe a case of bilateral renal cell carcinoma with the tumor thrombus in the inferior vena cava. A 71-year-old male was admitted to our department for, careful examination of bilateral renal tumors. Abdominal computed tomography (CT) revealed bilateral solid enhanced renal masses with the tumor thrombus in the inferior vena cava (IVC) and the left renal vein. The renal tumor did not reveal distant metastatic lesions. We enforced two-term operation for bilateral renal tumor. First, right nephrectomy and tumor thrombectomy in the IVC were performed. On the 32th day after the first operation, we performed left nephrectomy. On the second day after the last operation, the patient received hemodialysis. The histopathological diagnosis revealed bilateral renal cell carcinomas. At 9 months after the last operation, the patient had multiple bone metastases, multiple liver metastases and multiple lung metastases.     

老年患者双肾肿瘤的半保守治疗

目的:评价老年双肾肿瘤半保守治疗安全性。方法:对6例老年双肾肿瘤患者的临床资料进行回顾性分析。6例共12枚肿瘤,每例患者肿瘤均一大一小,影像学均高度怀疑肾癌。大肿瘤直径4.5~10.0cm,平均7.0cm均行根治性切除;小肿瘤直径1.8~2.7cm,平均2.3cm,予保守治疗。结果:手术均未发生严重的并发症,6例大肿瘤均为肾细胞癌:5枚混合细胞癌(以透明细胞为主型),分级Ⅱ级4枚、Ⅰ级1枚;1枚嫌色细胞癌,分级Ⅰ级。随访12~45个月,平均34个月,小肿瘤体积平均每年增大2.96(1.15~8.66)cm3,无局部浸润征象。手术侧未见复发,亦未见远处转移。结论:老年双肾肿瘤(一侧大肿瘤一侧小肿瘤)患者半保守治疗安全、有效。     

肾癌保留肾单位手术的临床价值(附17例报告)

目的　评价肾癌保留肾单位手术的临床价值。　方法　回顾性研究 17例行保留肾单位手术的肾癌患者临床资料 ,其中单侧 15例 ,双肾异时性肾癌且一侧为多发肿瘤 2例。绝对指征 2例 ,相对指征 6例 ,选择性指征 9例。肿瘤直径 2～ 6cm ,均为T1期 (1997年TNM标准 ) ;行改良肾肿瘤剜除术 (切缘于肾肿瘤外 1cm正常肾实质处 ) 15例 ,肾上极切除术 1例 ,楔形切除术 1例。 2 0例同期肾癌临床及分期相当但行根治性手术的患者资料作随访对照 ,并作生存率时序检验。　结果　 17例患者随访 3～ 6 3个月 ,平均 35 .2个月 ,未见并发症及残肾肿瘤复发。无瘤生存率与根治性手术者相近。　结论　肾癌保留肾单位手术安全、有效 ,适合于对侧肾功能正常、一侧局限的偶发肾肿瘤患者。     

遗传性肾癌11例临床分析

目的 探讨遗传性肾癌的诊断和治疗方法.方法 回顾性分析11例遗传性肾癌患者的临床资料,其中男8例、女3例,年龄32～67岁,平均48岁;4例为双侧肾癌,4例为多发肾癌.2例诊断为希佩尔-林道病综合征,6例诊断为家族性肾透明细胞癌,3例诊断为遗传性乳头状肾癌.10例患者行保留肾单位的手术和（或）肾癌根治术,1例未手术.结果 随访12～114个月,4例发生肿瘤复发,1例死于肿瘤转移,2例死于其他原因,4例无瘤生存.结论 遗传性肾癌发病年龄较早,肿瘤双侧、多中心发病率较高,应尽量行保留肾单位手术.     

Treatment of bilateral Wilms' tumor: comparison of initial biopsy and chemotherapy to initial surgical resection in the preservation of renal mass and function.

The National Wilms' Tumor Study 3 (NWTS-3) recommended treatment of bilateral Wilms' tumor with initial biopsy followed by chemotherapy with subsequent operation to resect the remaining tumor. This study was performed to determine if this approach preserves renal mass and function when compared with initial surgical resection followed by chemotherapy. Over a 20-year period (1970 to 1990), 15 patients with synchronous bilateral Wilms' tumor were treated at the Childrens Hospital of Los Angeles. Eight patients in the surgical group underwent initial unilateral nephrectomy with contralateral biopsy, wedge resection, or partial nephrectomy and subsequent chemotherapy. The seven patients in the chemotherapy group underwent bilateral tumor biopsy, followed by chemotherapy and subsequent tumor resection. Patients were assigned to each group in a nonrandomized manner according to the preference of the attending oncologist and surgeon. Comparison of the two groups showed no significant differences in sex distribution, initial renal function, tumor histology, dose and field of radiotherapy, presence or absence of positive surgical margins, and local recurrence rates. Patients in the surgery group were slightly older than those in the chemotherapy group: 3.6 +/- 2.2 versus 2.3 +/- 2.2 years. The percent of renal mass involved by tumor for the surgery group was 52 +/- 12 versus 73 +/- 16 for the chemotherapy group (P = .03). The percent of renal mass preserved following all operations, local recurrence rates, incidence of metastases, and survival was nearly identical between the two groups. There were three cases of renal failure in the surgery group and one case of renal failure in the chemotherapy group.(ABSTRACT TRUNCATED AT 250 WORDS)     

低龄婴儿双供肾移植给成人22例报道

目的总结单中心低龄婴儿双供肾移植给成人的临床效果。方法回顾性纳入2013年7月至2017年10月华中科技大学同济医学院附属同济医院实施的所有儿童双供肾移植给成人受者共22例临床资料和随访数据。22例供者年龄(2.9±1.7)个月,体重(4.9±1.4)kg,其中15例小于3月龄。受者多为低体重女性成人,体重(46.3±5.6)kg。总结早期移植失败及随访期间移植肾失功或受者死亡原因。根据是否发生单侧移植肾血栓,移植肾功能恢复者又进一步分为双肾存活组和单肾存活组,比较移植肾中-长期功能。结果4例受者在术后早期出现移植失败,包括双肾血栓2例、移植肾破裂切除1例和受者多器官功能衰竭死亡1例。18例受者移植肾功能恢复出院,随访期间因移植肾新生肿瘤切除双肾1例、因复杂全身原因死亡1例、因间质性肺炎死亡1例,余15例受者双肾均存活者10例(中位随访59个月),单肾存活者5例(中位随访48个月)。移植1年时双肾存活组估算肾小球滤过率为(95±27)ml/(min·1.73 m2),显著高于单肾存活组(61±24)ml/(min·1.73 m2)(P<0.05),但3年时分别为(95±21)ml/(min·1.73 m2)和(69±31)ml/(min·1.73 m2),差异缩小,差异无显著统计学意义(P=0.12)。结论低龄婴儿双供肾移植虽然可以扩大供肾来源,但发生早期移植失败和单肾栓塞的风险较高。在单肾存活的情况下,受者仍具有相对满意的中-长期移植效果。     

The role of renal salvage procedures for bilateral Wilms tumor: a 15-year review

PURPOSE: We reviewed our experience with renal salvage procedures in patients with bilateral Wilms tumor to determine the clinical outcome. MATERIALS AND METHODS: From 1982 to 1997, 23 children with bilateral Wilms tumor were treated with partial nephrectomy at our institution, including 7 who were also treated with brachytherapy. Medical history, use and response to chemotherapy and brachytherapy, operative records, renal function, pathological results, survival, and techniques for partial and repeat nephrectomy and brachytherapy were reviewed. RESULTS: We treated 8 boys and 15 girls, of whom 21 who presented with synchronous bilateral Wilms tumor underwent primary chemotherapy followed by secondary partial nephrectomy. A total of 44 partial nephrectomies were performed and brachytherapy was done in 7 patients. Ten children have normal renal function and no disease, 10 are dead and 2 have metastatic disease. Anaplasia was the most significant factor associated with an unfavorable outcome (p = 0.003). Of the patients who were cured 60% had a positive response to initial chemotherapy compared with only 25% who had an unfavorable outcome (p = 0.09). No significant differences were noted with respect to gender, age at presentation, highest local tumor stage at presentation or initial nephrectomy. No patient treated with brachytherapy had local recurrence. CONCLUSIONS: Preoperative chemotherapy followed by nephron sparing surgery is indicated in patients with bilateral Wilms tumor, while in those with diffuse anaplasia nephron sparing surgery is contraindicated. Brachytherapy should be considered for treating local disease involving chemoresistant tumors.     

A case of bilateral renal cell carcinoma showing a rapid course of death in a long-term hemodialysis patient due to polycystic kidney

A case of bilateral renal cell carcinoma in a 42-year-old polycystic kidney male is reported. He had been treated with hemodialysis for 22 years. An abnormal small mass was found in one of the left renal cystic lesions by screening ultrasonography and CT scan at the 19th year of the hemodialysis. Left radical nephrectomy was performed and the histological diagnosis was a renal cell carcinoma (RCC). There was no evidence of recurrence and metastasis, however, he presented with asymptomatic macrohematuria two years after the operation. CT scan demonstrated the rapidly progressing right renal tumor and multiple para-aortic lymph node swelling. Right nephrectomy and lymphadenectomy were performed and pathological examination showed the advanced RCC with multiple lymph node metastasis. Eleven months after the second operation followed by interferon therapy. he died of multiorgan metastasis of the RCC. This is the first bilateral RCC case in polycystic kidney patient treated with hemodialysis in Japan.     

Bilateral intrarenal pelvis Wilms' tumor with fibroepithelial polyp

Intrarenal pelvis Wilms' tumor is rare in children. A case of a 28-month-old boy with bilateral intrarenal pelvis Wilms' tumor associated with a fibroepithelial polyp is reported in this article. The tumor was evaluated by ultrasonography, computed tomography, and intravenous pyelography. The boy underwent bilateral renal pelviotomies. Now he is being treated and followed up by pediatric oncologists.     

肾癌肾部分切除术的临床价值及合适的手术切缘的探讨

目的：探讨肾癌肾部分切除术(保留肾单位手术)的临床价值及合适的手术切缘。方法：回顾性分析15例行肾部分切除术的肾癌患者临床资料．其中双侧异时性肾癌且一侧为多发肿瘤2例，单发肿瘤13例。肿瘤直径2～6cm．均为T1期(1997年TNM分期标准)。对15例肾癌患者行肾部分切除术．手术切缘位于肿瘤外1cm。另取肾癌根治性手术标本21例．于体外沿假包膜行肾肿瘤剜除术．并随机切取肿瘤边缘0．3cm、0．5cm及1cm处肾实质及肾蒂处淋巴脂肪组织行病理检查。结果：15冽患者随访12～72个月．平均41个月．未见并发症及残肾内肿瘤复发。21例标本于体外行肿瘤剜除后肉眼下均无肿瘤组织残留，送检组织均无肿瘤细胞浸润。结论：肾部分切除术能安全有效地治疗局限的早期肾癌患者．而手术切缘为肿瘤边缘1cm处较为合适。     

Conservative elective treatment of upper urinary tract tumors: a multivariate analysis of prognostic factors for recurrence and progression

PURPOSE: We evaluate the safety and efficacy of conservative elective treatment of upper urinary tract tumors, and determine predictive factors for recurrence and progression to optimize indications of this type of treatment. MATERIALS AND METHODS: Since 1984 we have performed a prospective study of conservative treatment of single, low grade and stage, less than 3 cm. upper tract tumors. The study includes 54 patients with a normal contralateral kidney who had been followed for more than 36 months. Open conservative surgery was performed in 31 cases and endourological surgery in 23. Minimum followup was 36 months, maximum 210 and mean 84.8. Univariate and multivariate analyses of recurrence and progression were performed in relation to age, sex, association with a bladder tumor, bladder tumor stage and grade, sequence of bladder tumor in relation to upper urinary tract tumor, number of previous bladder tumor recurrences, association with bladder carcinoma in situ, upper urinary tract tumor grade, stage, location, size and therapy, and upper urinary tract cytology. RESULTS: Of the 54 patients 19 (35%) had recurrence, which was bilateral recurrence in 4, and progression occurred in 9 (16%). At the end of analysis 44 (62.9%) patients were disease-free and alive at a mean time of 92.88 months, 13 (24%) died disease-free at a mean of 72.7 months and 7 (12.9%) died of disease at a mean of 97.85 months. Cause specific mortality occurred in 7 (12.9% cases). Among the 54 initially conservatively treated units 42 (77.7%) kidneys were ultimately preserved. On univariate and multivariate analysis tumor location in the renal pelvis and association with a previous multi-recurrent bladder tumor were variables significantly related to recurrence and progression, as well as bilateral recurrence. CONCLUSIONS: Conservative treatment is an optional approach for select upper urinary tract tumors. The strongest risk factors for recurrence and progression were association with a previous multi-recurrent bladder tumor and tumor location in the renal pelvis but these conditions were also the strongest risk factors for bilateral recurrence. Conservative treatment can also be recommended in these cases but only with compliant patients and close followup.     

Surgical management in synchronous bilateral renal cell carcinoma

Six patients with synchronous bilateral renal cell carcinoma were treated surgically over a 7-year period in our department. They were all males and the mean age was 60.3 years. They comprised 7.1% of all patients with renal cell carcinoma encountered during the same period. In four out of the six cases, radical nephrectomy for the larger tumor plus partial nephrectomy for contralateral kidney was performed simultaneously. In the remaining two cases, bilateral partial nephrectomy was performed simultaneously or as separate procedures. Two patients required chronic hemodialysis and died of cardio-pulmonary insufficiency on the 70th and 75th day. One patient, who underwent bilateral partial nephrectomy with incomplete tumor removal, subsequently died of metastatic disease at 27 months. Although one of them required transient hemodialysis, the remaining three patients were alive and disease-free 84, 42, and 17 months after operation, without evidence of tumor. This series suggests that partial nephrectomy is an appropriate option in the management of selected cases of bilateral renal cell carcinoma.     

Treatment of asynchronous bilateral renal cell carcinoma. On our experience of enucleation for two cases

In patients with either bilateral renal malignancies or with carcinoma occurring in a solitary kidney, the principle of en bloc removal of the tumor-bearing kidney cannot be applied. Recently we have performed surgical enucleation in two cases of asynchronous bilateral renal cell carcinoma. Case 1. A 60-year-old woman was hospitalized with diagnosis of left renal tumor 10 years tumor 10 years after right nephrectomy for renal cell carcinoma. The tumor was enucleated while occluding the renal vessels. Pathological examination revealed that the tumor (a nodule of 35 g) was renal cell carcinoma of grade I and perfectly covered by pseudocapsule. Hemodialysis was not required. The patient has been well for more than 11 months postoperatively and Ccr is 65 ml/min. Case 2. A 62-year-old man with slight elevation of serum GOT and GPT level was examined by CT, which revealed a space occupying lesion in the left kidney. He had undergone nephrectomy for renal cell carcinoma of right kidney 11 years ago. Three nodules of 56 g, 6 g and 3 g were removed by in situ enucleation. They were renal cell carcinoma of grade II and there was no malignant penetration of the pseudocapsule pathologically. After surgery hemodialysis was required 10 times for 21 days. Renal function has been refined gradually and the patient is well with 47.3 ml/min of Ccr at 4 months postoperatively. Before this report of 2 cases there were 22 cases of asynchronous bilateral renal cell carcinoma in Japanese literature.(ABSTRACT TRUNCATED AT 250 WORDS)